EWSR1-FUSION-NEGATIVE, SMARCB1-DEFICIENT PRIMARY PULMONARY MYXOID SARCOMA

Agaimy A, Duell T, Morresi-Hauf AT (2017)

Publication Type: Journal article

Publication year: 2017

Journal

Polish Journal of Pathology

Book Volume: 68

Pages Range: 261-267

Journal Issue: 3

DOI: 10.5114/PJP.2017.71535

Abstract

Primary pulmonary myxoid sarcoma (PPMS) is a recently defined rare neoplasm with histological and molecular similarity to extraskeletal myxoid chondrosarcoma. To date, 20 cases have been reported. A 48-year-old man presented with a huge mass filling the right hemithorax and extending into the tracheobronchial system. Histological findings were consistent with PPMS. Immunohistochemistry was positive for vimentin, CD10, and EMA, but other lineage-specific markers were negative. SMARCB1 (INI1) expression was lost in the tumour cells. FISH analysis (EWSR1, FUS, NR4A3, and SMARCB1) revealed no abnormalities. This case suggests SMARCB1 loss as a possible alternative molecular event driving EWSR1-negative PPMS.

Authors with CRIS profile

Abbas Agaimy Lehrstuhl für Allgemeine Pathologie und Pathologische Anatomie

Involved external institutions

Asklepios Fachkliniken München-Gauting DE Germany (DE)

How to cite

APA:

Agaimy, A., Duell, T., & Morresi-Hauf, A.T. (2017). EWSR1-FUSION-NEGATIVE, SMARCB1-DEFICIENT PRIMARY PULMONARY MYXOID SARCOMA. Polish Journal of Pathology, 68(3), 261-267. https://doi.org/10.5114/PJP.2017.71535

MLA:

Agaimy, Abbas, Thomas Duell, and Alicia T. Morresi-Hauf. "EWSR1-FUSION-NEGATIVE, SMARCB1-DEFICIENT PRIMARY PULMONARY MYXOID SARCOMA." Polish Journal of Pathology 68.3 (2017): 261-267.

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